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  • 1
    In: Rheumatology, 2017, Vol. 56(5), pp.853-854
    Keywords: Medicine;
    ISSN: 1462-0324
    E-ISSN: 1462-0332
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  • 2
    In: The New England Journal of Medicine, 2013, Vol.369(9), pp.866-868
    Description: Amyloidosis is a diverse group of diseases caused by extracellular accumulation of protein in a highly ordered, abnormal, insoluble fibrillar form. These diseases can be hereditary or acquired and localized or systemic. Most are progressive and fatal. Among the almost 30 different proteins that have been found to form amyloid in humans, transthyretin, a carrier molecule of thyroxine and vitamin A, is of particular interest because it may cause both hereditary and acquired amyloidosis with a diverse range of symptoms. In addition, transthyretin amyloid may be more prevalent than previously thought, and several potential new treatments are on the horizon. . . .
    Keywords: Europe ; Transthyretin ; Thyroxine ; Disease ; Amyloidosis ; Medical Research ; Proteins ; Vitamin A;
    ISSN: 0028-4793
    E-ISSN: 1533-4406
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  • 3
    Language: English
    In: Arthritis and rheumatism, February 2011, Vol.63(2), pp.314-24
    Keywords: Autoimmune Diseases -- Immunology ; Autoimmunity -- Immunology ; Immunity, Innate -- Immunology ; Interleukin-1beta -- Immunology
    E-ISSN: 1529-0131
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  • 4
    In: Kidney International, 2014
    Description: Amyloidosis results from protein misfolding, and ongoing amyloid deposition can ultimately lead to organ failure and death. Historically, this is a group of diseases with limited treatment options and frequently poor prognosis. However, there are now ‘targeted’ therapeutics emerging in the form of stabilizers of the precursor protein, inhibitors of fibrillogenesis, fibril disruptors, and blockers of protein translation, transcription, and immunotherapy. We review many of these approaches that are currently being assessed in clinical trials.
    Keywords: Amyloidosis ; Chemotherapy ; Diflunisal ; Eprodisate ; Immunotherapy ; Oligonucleotides ; Medicine;
    ISSN: 0085-2538
    E-ISSN: 15231755
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  • 5
    In: Rheumatology, 2011, Vol. 50(3), pp.624-626
    Keywords: Budd-Chiari Syndrome–Complications ; Child, Preschool–Genetics ; Cytoskeletal Proteins–Complications ; Familial Mediterranean Fever–Genetics ; Homozygote–Complications ; Humans–Complications ; Male–Complications ; Mutation–Complications ; Polyarteritis Nodosa–Complications ; Pyrin–Complications ; Abridged ; Cytoskeletal Proteins ; Mefv Protein, Human ; Pyrin;
    ISSN: 1462-0324
    E-ISSN: 1462-0332
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  • 6
    Article
    Article
    Language: English
    In: Best Practice & Research Clinical Rheumatology
    Description: Periodic fever syndromes are autoinflammatory diseases. The majority present in infancy or childhood and are characterised by recurrent episodes of fever and systemic inflammation that occur in the absence of autoantibody production or identifiable infection. The best recognised disorders include CAPS, FMF, TRAPS and MKD. Understanding the molecular pathogenesis of these disorders provides unique insights into the regulation of innate immunity. Diagnosis relies on clinical acumen and is supported by genetic testing. With the exception of FMF, which is prevalent in populations originating from the Mediterranean, these syndromes are rare and easily overlooked in the investigation of recurrent fevers. Disease severity varies from mild to life threatening, and one of the most feared complications is AA amyloidosis. Effective therapies are available for many of the syndromes, including colchicine, IL-1 blockade and anti-TNF therapies, and there is an increasing interest in blocking interferon pathways.
    Keywords: Cryopyrin-Associated Periodic Syndromes (Caps) ; Familial Mediterranean Fever (Fmf) ; Tumour Necrosis Factor (Tnf) Receptor-Associated Periodic Syndrome (Traps) ; Mevalonate Kinase Deficiency (Mkd) ; Deficiency of Il-1 Receptor Antagonist (Dira) ; Deficiency of Il-36 Receptor Antagonist (Ditra) ; Blau Syndrome ; Chronic Atypical Neutrophilic Dermatosis With Lipodystrophy and Elevated Temperature Syndrome (Candle) ; Deficiency of Adenosine Deaminase 2 (Dada2) ; Aphthous Stomatitis ; Pharyngitis and Cervical Adenitis (Pfapa) ; Medicine
    ISSN: 1521-6942
    E-ISSN: 1532-1770
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  • 7
    In: Journal of Translational Medicine, 2011, Vol.9(Suppl 2), p.I6-I6
    Description: The inherited periodic fever syndromes are single gene disorders of innate immunity which have provided invaluable insights into the regulation of inflammation. They are all extremely rare but are clinically important as they cause very significant day to day symptoms and carry a high risk of early death due to AA amyloidosis. With the exception of familial Mediterranean fever treatment was essentially ineffective until this century.
    Keywords: Invited Lecture Presentation
    E-ISSN: 1479-5876
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  • 8
    Language: English
    In: Clinical medicine (London, England), June 2015, Vol.15(3), pp.295-8
    Description: The systemic autoinflammatory syndromes often present with recurrent fevers. They have proved exceptionally informative about the innate immune system. Although extremely rare, they are important to recognise, as many can now be completely controlled by long-term drug therapies. Diagnosis relies on clinical suspicion followed by genetic testing.
    Keywords: Autoinflammation ; Caps ; Fmf ; Il-1 ; Mkd ; Pfapa ; Schnitzler'S Syndrome ; Traps ; Systemic Autoinflammatory Syndromes ; Fever of Unknown Origin -- Etiology ; Hereditary Autoinflammatory Diseases -- Complications
    ISSN: 1470-2118
    E-ISSN: 14734893
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  • 9
    In: Rheumatology, 2011, Vol. 50(7), pp.1349-1350
    Keywords: Medicine;
    ISSN: 1462-0324
    E-ISSN: 1462-0332
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  • 10
    In: Neurology, 2010, Vol.74(16), pp.1267-1270
    Description: BACKGROUND:: The cryopyrin-associated periodic syndrome (CAPS) is a rare but treatable hereditary autoinflammatory condition. Without treatment, one third of patients develop amyloidosis with consequent renal failure and death. CAPS encompasses 3 conditions: familial cold autoinflammatory syndrome, Muckle–Wells syndrome, and chronic infantile, neurologic, cutaneous, and articular syndrome. Neurologic complications are common in children with the chronic infantile, neurologic, cutaneous, and articular phenotype, but there are no previous published reports of neurologic features in adults with milder phenotypes. METHODS:: In this case series, we report in detail an adult case of CAPS and summarize the neurologic features seen in 12 other adults with genetically proven CAPS. These patients participated in a recent randomized study of canakinumab in CAPS and we used pretreatment data collected in this study. RESULTS:: Twelve of the 13 patients (92%) had headache, of whom 10 (77%) had features of migraine. Seven patients (54%) had sensorineural deafness. Nine patients (69%) reported myalgia. Six patients (46%) had papilledema and a further 2 (15%) had optic disc pallor. MRI brain scan was normal in all patients. CONCLUSION:: CAPS is a rare but treatable condition that may be encountered by neurologists in adult clinical practice since it can present with headache, myalgia, papilledema, sensorineural deafness, and aseptic meningitis. Unrecognized and untreated, it can lead to significant morbidity and mortality from renal failure. Treatment with anti-interleukin-1 therapy leads to complete resolution of symptoms and should also prevent progression to amyloidosis and subsequent renal failure. GLOSSARY:
    Keywords: Optics ; Mortality ; Aseptic Meningitis ; Data Processing ; Magnetic Resonance Imaging ; Brain ; Renal Failure ; Neurological Complications ; Children ; Swelling ; Morbidity ; Optic Nerve ; Deafness ; Amyloidosis ; Migraine ; Headache ; Myalgia ; Neurology & Neuropathology;
    ISSN: 0028-3878
    E-ISSN: 1526632X
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